Evidence map›Paper›PMID 40940654›Full record

ArticleNeurological research and practice2025

Real-world treatment management in hereditary transthyretin amyloidosis - an experience report and proposal for therapy switch decision criteria.

Duc Chu Dieu, Helena F Pernice, Harisa Muratovic, Paul J Wetzel, Gina Barzen, Nicolas W Wieder, Stefanie M Werhahn, Bettina Heidecker, Sebastian Spethmann, Katrin Hahn

Abstract read
In one paragraph

Article in Neurological research and practice, 2025. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 1 paper.

0numbers the graph read from it
0cells of the map it votes in
1citing papers in PubMed
–field-weighted citation impact
1 · What the graph read from it

What it found

Each row is one number read from the abstract, on the scale the paper reported it, with its interval. Left of the dashed line favours the treatment, right favours the comparator. Under each row is the sentence it came from. New to these charts? A ten-minute tutorial.

The abstract states no effect estimate the extractor could read, or names no intervention and outcome on the map, so this paper lights no cell and moves no belief. It is still indexed, cited and linked below.

2 · The registry

The trial behind it

Trials whose registry record cites this paper, or whose number appears in the abstract. A trial that started after this paper was published is citing it as background, not reporting it.

Neither the registry nor the abstract names a trial number. If this is a trial report, that itself is worth knowing.

3 · Its place in the literature

Who cites it

1 citing paper in PubMed.

  1. Review
4 · The record

Corrections and comments

PubMed lists nothing against this paper. Absence here is not a guarantee, only a check that was made.

5 · Who and what money

Authors and funding

10 authors.

Duc Chu Dieu *Amyloidosis Center Charité Berlin (ACCB), Charité University Medicine, Berlin, Germany.ORCID http://orcid.org/0009-0008-4929-5790
Helena F Pernice *Amyloidosis Center Charité Berlin (ACCB), Charité University Medicine, Berlin, Germany.ORCID http://orcid.org/0009-0001-8711-5526
Harisa MuratovicAmyloidosis Center Charité Berlin (ACCB), Charité University Medicine, Berlin, Germany.ORCID http://orcid.org/0009-0008-5170-3722
Paul J WetzelAmyloidosis Center Charité Berlin (ACCB), Charité University Medicine, Berlin, Germany.ORCID http://orcid.org/0009-0001-9393-5512
Gina BarzenAmyloidosis Center Charité Berlin (ACCB), Charité University Medicine, Berlin, Germany.ORCID http://orcid.org/0000-0002-1329-2159
Nicolas W WiederAmyloidosis Center Charité Berlin (ACCB), Charité University Medicine, Berlin, Germany.ORCID http://orcid.org/0000-0001-5683-2891
Stefanie M WerhahnAmyloidosis Center Charité Berlin (ACCB), Charité University Medicine, Berlin, Germany.ORCID http://orcid.org/0000-0002-0805-3129
Bettina HeideckerAmyloidosis Center Charité Berlin (ACCB), Charité University Medicine, Berlin, Germany.ORCID http://orcid.org/0000-0002-3811-7920
Sebastian SpethmannAmyloidosis Center Charité Berlin (ACCB), Charité University Medicine, Berlin, Germany.ORCID http://orcid.org/0000-0003-0987-8007
Katrin HahnAmyloidosis Center Charité Berlin (ACCB), Charité University Medicine, Berlin, Germany. katrin.hahn@charite.de.ORCID http://orcid.org/0000-0002-6013-0072

Funding

No grant is acknowledged in the PubMed record.

6 · The paper itself

Abstract

backgroundHereditary transthyretin amyloidosis is a rapidly progressive and lethal disease. Thanks to the increasing number of disease-modifying treatments, prognosis has improved significantly. However, new challenges regarding treatment response and when to change treatment remain unanswered. The objective of this study was to evaluate rationales for treatment switches from the past and to formulate learnings for future management.

methodsIn this retrospective single center study, we analyzed real-world data of 13 patients with hereditary transthyretin amyloidosis undergoing single or multiple treatment switches before January 2024. Data involved demographic characteristics as well as reasons for treatment switches in a descriptive and exploratory manner. Available amyloid specific therapies during the study period included tafamidis 20 mg, tafamidis 61 mg, patisiran, inotersen and vutrisiran.

resultsSwitches from tafamidis 20 mg were most frequently due to disease progression (83.3%). Patisiran transitions predominantly occurred following vutrisiran's approval, driven by preference for subcutaneous administration and extended dosing intervals (65.0%). Two cases of switches from inotersen were both associated with severe adverse effects.

conclusionsIn this study, reasons for treatment switches were manifold, encompassing disease progression, the occurrence of adverse events, patient preferences and/or the availability of newly approved drugs. Hence, multidimensional consideration of these reasons remains pivotal in guiding the subsequent choice of medication in particular and managing hereditary transthyretin amyloidosis in general.

Indexed as

ASOGene silencerHereditary transthyretin amyloidosisInotersenPatisiranSiRNATafamidisTetramer stabilizerVutrisiran

Identifiers

PMID40940654
PMCPMC12432994

What Socratic holds

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Registered trials

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Read under generation 80e0d062 · epoch 390. Bibliography from PubMed, PubMed Central and OpenAlex; grants from NIH RePORTER; trial links from ClinicalTrials.gov; estimates, votes and beliefs from the Socratic graph.